Fetoscopic myelomeningocoele closure: is the scientific evidence enough to challenge the gold standard for prenatal surgery?

Since the completion of the Management of Myelomeningocoele Study, maternal-fetal surgery for spina bifida has become a valid option for expecting parents. More recently, multiple groups are exploring a minimally invasive approach and recent outcomes have addressed many of the initial concerns with this approach. Based on a previously published framework, we attempt to delineate the developmental stage of the surgical techniques. Furthermore, we discuss the barriers of performing randomized controlled trials comparing two surgical interventions and suggest that data collection through registries is an alternative method to gather high-grade evidence.Research into fetal development and medicin

Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: The CDH EURO Consortium Consensus - 2015 Update

In 2010, the congenital diaphragmatic hernia (CDH) EURO Consortium published a standardized neonatal treatment protocol. Five years later, the number of participating centers has been raised from 13 to 22. In this article the relevant literature is updated, and consensus has been reached between the members of the CDH EURO Consortium. Key updated recommendations are: (1) planned delivery after a gestational age of 39 weeks in a high-volume tertiary center; (2) neuromuscular blocking agents to be avoided during initial treatment in the delivery room; (3) adapt treatment to reach a preductal saturation of between 80 and 95% and postductal saturation >70%; (4) target PaCO2 to be between 50 and 70 mm Hg; (5) conventional mechanical ventilation to be the optimal initial ventilation strategy, and (6) intravenous sildenafil to be considered in CDH patients with severe pulmonary hypertension. This article represents the current opinion of all consortium members in Europe for the optimal neonatal treatment of CDH

Fetoscopic surgery for in utero management of congenital diaphragmatic hernia

Until recently two possibilities were available to the expectant parents of a fetus diagnosed with a congenital diaphragmatic hernia: termination of pregnancy or continuation of the pregnancy until term with a potential change in the place of delivery. Open fetal surgery has been used to treat a growing number of congenital malformations with life-threatening or highly morbid consequences including congenital diaphragmatic hernia. However, its effectiveness is limited by the occurrence of preterm labour, chorioamniotic membrane separation, preterm prelabour rupture of the membranes and altered fetal homeostasis. These problems were the impetus for the development of minimal access fetal surgery. Developments in endoscopic surgical technology over the past three decades have provided the opportunity to develop techniques adapted for prenatal fetal intervention

Absence of electrical uterine activity after endoscopic access for fetal surgery in the Rhesus monkey

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Reply: "Prenatal Prediction of Pulmonary Hypoplasia: US and MR Imaging Working Together"

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Evidence and patterns in lung response after fetal tracheal occlusion: Clinical controlled study

Purpose: To prospectively assess changes in lung volume in fetuses with isolated severe congenital diaphragmatic hernia (CDH) after fetoscopic endoluminal tracheal occlusion (FETO) compared with those in fetuses with CDH of variable severity who were expectantly managed. Materials and Methods: Informed consent was obtained for this ethics committee-approved study. Forty fetuses with severe CDH (lung-to-head ratio < 1 and intrathoracic liver) who underwent FETO and 18 fetuses with CDH of variable severity who were expectantly managed were longitudinally followed up by using magnetic resonance (MR) imaging volume measurements. Fetuses born prior to 32 weeks were excluded from the study. For those undergoing FETO, lung volume responsiveness was defined as the proportionate increase in observed-to-expected (O/E) ratio of total fetal lung volume (TFLV) at 2-5 weeks after FETO compared with the pre-FETO value. Changes in lung volume were compared by using the Mann-Whitney U test. Regression analysis was used to investigate the effect of pre-FETO O/E ratio of TFLV, gestational age at FETO and at delivery, lung volume responsiveness, occlusion period, side of CDH, and balloon removal prior to delivery on survival. Correlation between post-FETO lung volume responsiveness and gestational age at FETO was performed by using linear regression analysis. Results: A total of 260 MR imaging examinations were performed. For expectantly managed fetuses, O/E ratio of TFLV remained unchanged during gestation, whereas it significantly increased after FETO. Regression analysis demonstrated that pre-FETO O/E ratio of TFLV and lung volume responsiveness at 3.3 weeks after FETO provided significant independent prediction of postnatal survival. There was a significant negative association between lung volume responsiveness and gestational age at FETO. Conclusion: In fetuses with CDH, pre-FETO O/E ratio of TFLV and lung volume at 3.3 weeks after FETO provide independent prediction of postnatal survival. © RSNA, 2009.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Fetal Body Volume at MR Imaging to Quantify Total Fetal Lung Volume - Normal Ranges

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Lung volumes in fetuses with diaphragmatic hernia: comparison of 3D US and MR imaging assessments

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Prenatal prediction of survival in isolated diaphragmatic hernia using observed to expected total fetal lung volume determined by magnetic resonance imaging based on either gestational age or fetal body volume

Objectives: To compare the predictive value of the prenatal observed to expected (o/e) lung volume as measured by fetal magnetic resonance imaging (MRI), based on an algorithm using either the gestational age or fetal body volume (FBV), for neonatal survival of fetuses with isolated congenital diaphragmatic hernia (CDH). Methods: We included 53 fetuses with a prenatal diagnosis of isolated CDH, 26 without and 27 with prenatal tracheal occlusion, who were assessed by fetal MRI, liveborn after 32 weeks, and in whom follow-up until discharge from the neonatal care unit was available. Measurements of lung volumes were expressed as a percentage of the appropriate mean (o/e total fetal lung volume (TFLV) x 100) either for gestational age or for FBV. Measurements of FBV were expressed as a percentage of the appropriate mean (o/e FBV x 100) for gestation. Fetuses with prenatal intervention were all assessed ≥24 h after balloon removal. Regression analysis was used to examine the effect on postnatal survival of either o/e TFLV based on gestational age or based on FBV, gestation at delivery, side of CDH, intrathoracic position of the liver and prenatal intervention. Receiver-operating characteristics (ROC) curves were constructed for the prediction of survival by o/e TFLV based on gestational age and o/e TFLV based on FBV, for all fetuses, as well as for those with o/e FBV between 90 and 110% and those with values beyond that range. A power calculation for the number of fetuses needed to show a difference between the ROC curves was performed. Results: Regression analysis demonstrated that o/e TFLV based on gestational age and on FBV were the only independent predictors of postnatal survival. The area under the ROC curve for prediction of postnatal survival from the o/e TFLV based on gestational age was 0.811, and for that based on FBV it was 0.868 (P 110%, the area for measurements based on gestational age was 0.895 and 0.733, respectively; when based on FBV it was 0.906 and 0.833 (P < 0.01 for all). A minimum of 273 patients would be needed to provide a probability of 90% of detecting a difference between the areas under both ROC curves. Conclusions: In fetuses with isolated CDH, lung volume as measured by fetal MRI was significantly correlated with survival. Prediction tended to be better by o/e TFLV based on FBV rather than gestational age. The difference in the prediction of survival between o/e TFLV based on FBV or gestational age was dependent on fetal biometry. Copyright © 2008 ISUOG. Published by John Wiley & Sons, Ltd.SCOPUS: ar.jFLWINinfo:eu-repo/semantics/publishe

Quantification of intrathoracic liver herniation by magnetic resonance imaging and prediction of postnatal survival in fetuses with congenital diaphragmatic hernia

Objective: To quantify the degree of intrathoracic liver herniation by magnetic resonance imaging (MRI) and evaluate its effect on postnatal survival in fetuses with isolated congenital diaphragmatic hernia (CDH). Methods: Forty fetuses that were expectantly managed and that were delivered after 32 weeks' gestation were included in this study. On axial T2 weighted MR images the degree of intrathoracic liver herniation was measured by volumetry, using the xyphoid process and thoracic apex as landmarks. The ratio of the volume of the liver that was herniated into the thoracic cavity to the volume of the thoracic cavity was calculated (LiTR). All the fetuses also underwent lung volumetry, and the ratio of the observed/expected total fetal lung volume (o/e TFLV) was calculated. Regression analysis was used to investigate the effect on survival of side of occurrence of CDH, o/e TFLV, LiTR and gestational age at delivery. Receiver-operating characteristics (ROC) curves were constructed to examine the prediction of survival by o/e TFLV or LiTR alone and o/e TFLV and LiTR together. Results: Univariate regression analysis demonstrated that significant predictors of survival were o/e TFLV and LiTR. Multiple regression analysis demonstrated that o/e TFLV and LiTR provided independent prediction of survival. The area under the ROC curve (AUC) for the prediction of postnatal survival from o/e TFLV alone was 0.846 (P < 0.001; SE = 0.062) and the AUC from LiTR alone was 0.875 (P = 0.001; SE = 0.072). The AUC for the prediction of postnatal survival from o/e TFLV and LiTR combined was 0.912 (P < 0.001; SE = 0.045), however it was not statistically significantly different from that of o/e TFLV alone. Conclusion: In expectantly managed CDH fetuses, assessment of LiTR using MRI provided prediction of postnatal survival independently from o/e TFLV. Copyright © 2008 ISUOG. Published by John Wiley & Sons, Ltd.SCOPUS: ar.jFLWINinfo:eu-repo/semantics/publishe